Primary Lymphedema: Clinical Features and Management in 138 Pediatric Patients

Schook, Carolyn C.; Mulliken, John B.; Fishman, Steven J.; Grant, Frederick D.; Zurakowski, David; Greene, Arin K.

doi:10.1097/prs.0b013e318213a218

Cited by 140 publications

(144 citation statements)

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“…Less commonly, lymphedema occurs as a result of congenital causes. [1][2][3][4] Surgical procedures to treat lymphedema have existed for many years. Vascularized lymph node transfer (VLNT) is a microsurgical procedure that involves the relocation of a lymphatic-containing soft tissue flap along with its arteriovenous supply from a donor site such as the lateral groin, chest wall, or neck to the affected limb, groin, or axilla.…”

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confidence: 99%

An Effective System of Surgical Treatment of Lymphedema

et al. 2014

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Background. Effective surgical treatments for lymphedema now can address the fluid and solid phases of the disease process. Microsurgical procedures, including lymphaticovenous anastomosis (LVA) and vascularized lymph node transfer (VLNT), target the fluid component that predominates at earlier stages of the disease. Suctionassisted protein lipectomy (SAPL) addresses the solid component that typically presents later as chronic, nonpitting lymphedema of an extremity. We assess the outcomes of patients who underwent selective application of these three surgical procedures as part of an effective system to treat lymphedema. Methods. This is a retrospective chart review of patients with lymphedema who underwent complete decongestive therapy followed by surgical treatment with SAPL, LVA, or VLNT. The primary outcomes measured were postoperative volume reduction (SAPL), daily requirement for compression garments and lymphedema therapy (VLNT and LVA), and the incidence of severe cellulitis. Results. Twenty-six patients were included in the study, of which 10 underwent SAPL and 16 underwent LVA or VLNT. The average reduction of excess volume by SAPL was 3,212 mL in legs and 943 mL in arms, or a volume reduction of 87 and 111 %, respectively, when compared with the unaffected, opposite sides. Microsurgical procedures (VLNT and LVA) significantly reduced the need for both compression garment use (p = 0.003) and lymphedema therapy (p \ 0.0001). The overall rate of cellulitis decreased from 58 % before surgery to 15 % after surgery (p \ 0.0001).Conclusions. When applied appropriately to properly selected patients, surgical procedures used in the treatment of lymphedema are effective and safe.Lymphedema in the developed world commonly occurs after lymph node dissection and/or radiotherapy to nodal basins for breast cancer, melanoma, or gynecologic malignancies. Less commonly, lymphedema occurs as a result of congenital causes.

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An Effective System of Surgical Treatment of Lymphedema

et al. 2014

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“…Even among patients who undergo isolated axillary sentinel lymph node biopsy, up to 7 % have measurable arm differences, and up to 10 % have subjective symptoms of lymphedema. [1][2][3][4][5][6][7][8] Acquired lymphedema results from the accumulation of lymphatic fluid in the affected limb after interruption of normal lymphatic drainage channels. Initially, swelling is due to excess lymphatic fluid and is characterized by pitting edema.…”

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confidence: 99%

Review of Current Surgical Treatments for Lymphedema

et al. 2014

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Background. The current mainstay of lymphedema therapy has been conservative nonsurgical treatment. However, surgical options for lymphedema have been reported for over a century. Early surgical procedures were often invasive and disfiguring, and they often had only limited long-term success. In contrast, contemporary surgical techniques are much less invasive and have been shown to be effective in reducing excess limb volume, the risk of cellulitis, and the need for compression garment use and lymphedema therapy. Microsurgical procedures such as lymphaticovenous anastomosis and vascularized lymph node transfer lymphaticolymphatic bypass can treat the excess fluid component of lymphedema swelling that presents as pitting edema. Suction-assisted protein lipectomy is a minimally invasive procedure that addresses the solid component of lymphedema swelling that typically occurs later in the disease process and presents as chronic nonpitting lymphedema. These surgical techniques are becoming increasingly popular and their success continues to be documented in the medical literature. We review the efficacy and limitations of these contemporary surgical procedures for lymphedema. Methods. A Medline literature review was performed of lymphedema surgery, vascularized lymph node transfer, lymphaticovenous anastomosis, lymphatic liposuction, and lymphaticolymphatic bypass with particular emphasis on developments within the past 10 years. A literature review of technique, indications, and outcomes of the surgical treatments for lymphedema was undertaken.Results. Surgical treatments have evolved to become less invasive and more effective. Conclusions. With proper diagnosis and the appropriate selection of procedure, surgical techniques can be used to treat lymphedema safely and effectively in many patients when combined with integrated lymphedema therapy.Lymphedema is a progressive and debilitating condition associated with dysfunction of the lymphatic system. While a small percentage of cases are congenital, most patients in developed countries present with lymphedema resulting from treatment of malignancy. The true incidence of lymphedema is difficult to determine as a result of significant differences in diagnostic criteria. However, lymphedema is reported to occur in up to 49 % of breast, 20 % of gynecologic, 16 % of melanoma, 10 % of genitourinary, and 6 % head and neck cancer patients after lymph node dissection and/or radiotherapy. Even among patients who undergo isolated axillary sentinel lymph node biopsy, up to 7 % have measurable arm differences, and up to 10 % have subjective symptoms of lymphedema.

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“…The progression and morbidity is not influenced by the age of onset [1]. It affects males and females equally.…”

Section: Discussionmentioning

confidence: 99%

“…Genitalia and lower extremities are the most frequent locations affected by this pathology. Complications include infection, functional disability, chronic cutaneous changes and psychosocial morbidity [1][2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%